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Manuscript Accepted Early View Article
Page 1 of 13
Early View Article: Online published version of an accepted article before publication in the final form.
Journal Name: International Journal of Hepatobiliary and Pancreatic Diseases (IJHPD)
Type of Article: Case Report
Title: Embryonal Rhabdomyosarcoma of liver in a 16 year old male - Rare case report
Authors: Rajamahendran Rajendran, Amudhan A, Prabhakaran R, Kannan D,
Chandramohan SM
doi: To be assigned
Received: 11th August 2014
Accepted: 3rd September 2014
How to cite the article: Rajendran R, Amudhan A, Prabhakaran R, Kannan D, Chandramohan SM. Embryonal Rhabdomyosarcoma of liver in a 16 year old male - Rare case report. International Journal of Hepatobiliary and Pancreatic Diseases (IJHPD). Forthcoming 2014.
Disclaimer: This manuscript has been accepted for publication. This is a pdf file of the Early View Article. The Early View Article is an online published version of an accepted article before publication in the final form. The proof of this manuscript will be sent to the authors for corrections after which this manuscript will undergo content check, copyediting/proofreading and content formatting to conform to journal’s requirements. Please note that during the above publication processes errors in content or presentation may be discovered which will be rectified during manuscript processing. These errors may affect the contents of this manuscript and final published version of this manuscript may be extensively different in content and layout than this Early View Article.
Manuscript Accepted Early View Article
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TYPE OF ARTICLE: Case Report 1
2
TITLE: Embryonal Rhabdomyosarcoma of liver in a 16 year old male - Rare case 3
report 4
5
AUTHORS: 6
Rajamahendran Rajendran1, Amudhan A2, Prabhakaran R3, Kannan D4, 7
Chandramohan SM5 8
9
AFFILIATIONS: 10
1Post Graduate in Surgical Gastroenterology, Institute of Surgical Gastroenterology, 11
Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India. Email ID: 12
2Assistant Professor in Surgical Gastroenterology, Institute of Surgical 14
Gastroenterology, Rajiv Gandhi Government General Hospital, Chennai, Tamil 15
Nadu, India. Email ID: [email protected] 16
3Assistant Professor in Surgical Gastroenterology, Institute of Surgical 17
Gastroenterology, Rajiv Gandhi Government General Hospital, Chennai, Tamil 18
Nadu, India. Email ID: [email protected] 19
4Professor of Surgical Gastroenterology, Institute of Surgical Gastroenterology, Rajiv 20
Gandhi Government General Hospital, Chennai, Tamil Nadu, India. Email ID: 21
5Head of Department of Surgcial Gastroenterology, Institute of Surgical 23
Gastroenterology, Rajiv Gandhi Government General Hospital, Chennai, Tamil 24
Nadu, India. Email ID: [email protected] 25
26
CORRESPONDING AUTHOR DETAILS 27
Dr. Rajamahendran Rajendran, 28
No. 245, Tower 2, 4th Floor, Rajiv Gandhi Government General Hospital, Chennai-29
600003, Tamil Nadu, India. 30
Contact Phone Number: 919787387183 31
Contact Email ID: [email protected] 32
Manuscript Accepted Early View Article
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Short Running Title: 33
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Guarantor of Submission: The corresponding author is the guarantor of 35
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Manuscript Accepted Early View Article
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TITLE: Embryonal Rhabdomyosarcoma of liver in a 16 year old male - Rare case 64
report 65
66
ABSTRACT 67
Introduction: 68
Primitive mesenchymal tumors represent about 9%-15% of all hepatic tumors in 69
children. Only about 150 cases have been reported in the literature so far. Hereby 70
we report a case of Ruptured Embryonal Rhabdomyosarcoma. 71
72
Case Report: 73
A boy aged 16 years old with weight of 26 kg presented to us with sudden onset of 74
abdominal pain and fever. Clinical examination revealed a tender hepatomegaly 75
about 6 cm below Right costal margin. CECT abdomen revealed 15x 11x 9 cm 76
heterogenous multi septated loculated mass lesion involving right lobe of liver 77
involving segments 5,6,7,8. Diagnostic laparoscopy was done which showed a tumor 78
occupying the entire right lobe of liver with no other deposits. The tumor capsule was 79
seen ruptured on the right lateral side for which we did emergency right 80
Hepatectomy. Weight of the specimen was 2.8 kilograms. 81
Histopathology revealed the lesion as Embryonal Rhadomyosarcoma with co-82
existing mesenchymal hamartoma. Immunohistochemistry showed Vimentin and 83
Desmin positive. Patient was on carboplatin and Ifosfamide chemotherapy. Patient 84
expired on 30th postoperative day due to acute respiratory distress. 85
86
Conclusion: 87
Improved survival can be expected only if we detect the disease early. This case 88
report will enlighten this type of rare tumor in young children and an early referral to 89
the specialist. 90
91
Keywords: Primary Embryonal Rhabdomyosarcoma liver, Heterogenous mass in 92
liver, Ruptured capsule, Right Hepatectomy. 93
94
Manuscript Accepted Early View Article
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TITLE: Embryonal Rhabdomyosarcoma of liver in a 16 year old male - Rare case 95
report 96
97
INTRODUCTION 98
Primary liver tumors rank the third amongst the solid malignant tumors in children 99
following Wilms tumor and Neuroblastoma. They account for 2% of total solid 100
malignancies in paediatric cases [1]. Primitive mesenchymal tumors rank the fourth 101
among the malignant tumors of paediatric age group following Hepatoblastoma, 102
Infantile hemangio endothelioma and Hepatocellular carcinoma. 103
Rhabdomyosarcoma is the malignant solid tumor arising from mesenchymal tissues 104
which normally differentiates to form striated muscle. It is most common tumor in the 105
age group less than 15 years. There are four types of Rhabdomyosarcoma-106
Pleomorphic, Embryonal, Alveolar and Botryoidal. The 5 year survival rate of 107
localised disease is 80% and for metastatic disease- 5% 108
Primitive mesenchymal tumors represent about 9%-15% of all hepatic tumors in 109
children. So far only 150 cases of rhabdomyosarcoma liver have been reported in 110
the literature. Hereby we report a case of ruptured primary Embryonal 111
Rhabdomyosarcoma of liver in a 16 year old male boy. Embryonal type of 112
Rhabdomyosarcoma of liver is very rare and only 12 cases were reported in the 113
literature so far. 114
115
CASE REPORT 116
16 years male with body weight of 26 kilograms presented to us with a mass in the 117
right hypochondrium past 1 month with history of abdominal pain, fever with chills 118
and rigors. He had no history of jaundice, weight loss or loss of appetite. On 119
elaborating the history, the mass was present for the past 1 month and rapidly 120
increased for the past 1 week with severe intolerable pain past 4 days. On clinical 121
examination abdomen was soft and minimal guarding was present. A firm tender 122
heptomegaly was present 6 cm below right costal margin. Surface found to be 123
smooth and edges were rounded. CECT abdomen revealed 15x 11x 9 cm hetero 124
genous multi septated loculated mass lesion involving right lobe of liver involving the 125
segments 5,6,7,8 (Figure 1 and 2). Portal venous Doppler study showed displaced 126
Manuscript Accepted Early View Article
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and stretched right portal vein with normal flow. Laboratory investigations at the time 127
of admission were Hemoglobin 8 gm/dl, Platelets- 4 lakhs/cubic mm, Blood sugar – 128
80 mg/dl, Blood urea- 22 mg/dl and Serum creatinine- 0.8 mg/dl. Two units of 129
packed cells were transfused and the preoperative hemoglobin was 10 mg/dl. Tumor 130
markers AFP and CA 19-9 were done. Alpha fetoprotein value was 4 ng/ml and CA 131
19-9 was found to 20 U/ml. Liver biopsy was not taken to avoid the risk of seedling of 132
an operable tumor. 133
Diagnostic laparoscopy revealed a tumor occupying the segments 5,6,7,8 with 134
ruptured capsule in the right lateral lobe of liver (Figure 3). There was no other 135
peritoneal or omental mets. The left lobe of liver was found normal with other lesions. 136
So we decided to proceed with right hepatectomy. The inflow control was done with 137
Pringles manoeuvre. Parenchymal dissection was done with Harmonic scalpel and 138
Kelly crush clamp technique (Figure 4, 5). The bleeding vessels from raw area in the 139
left lobe were ligated with 3’0 Silk sutures. Specimen retrieved and complete 140
haemostasis was achieved in the remnant liver raw area. Weight of the specimen 141
was 2.8 kilograms (Figure 6). 142
Histopathology showed liver parenchyma with tumor composed of round to 143
polyhedral and spindle shaped cells with bizarre nuclei, arranged in sheets and 144
clusters with vague lobular pattern in some places. Tumor cells inhibit 145
intracytoplasmic globules and multinucleate giant cells with atypical mitosis. 146
Entrapped bile ductules are seen. Stroma shows extensive haemorrhagic areas with 147
areas of necrosis. The above features are suggestive of Embryonal variety of 148
Rhabdomyosarcoma probably coexisting with mesenchymal hamartoma (Figure 7 a, 149
7b), resected margins were free of tumor. 150
IHC markers are positive for Vimentin showed focal positivity, Desmin showed 151
diffuse strong positivity, CK 7 glands showed positivity. CD 34 was found negative. 152
All these confirmed the diagnosis of Embryonal variety of Rhabdomyosarcoma. 1st 153
cycle of chemotherapy started with Ifosfamide at 800 mg/day and Carboplatin at 300 154
mg/day. 155
Patient expired on 30th postoperative day suddenly because of respiratory distress 156
and pleural effusion. Post mortem examination was not done. 157
158
Manuscript Accepted Early View Article
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DISCUSSION 159
Having an incidence of 9-15% in children, Hepatic Rhabdomyosarcoma liver needs a 160
lot of experience to diagnose. Rhadomyosarcoma was classified into four types- 161
Pleomorphic, Alveolar, Embryonal and Botryoidal types. The predominant age of 162
presentation are Pleomorphic type in male adults, Alveolar type in adolescents and 163
young adults, Embryonal type in infants and children and Botryoidal type in young 164
children. The different types of Rhabdomyosarcoma can be differentiated on the 165
basis of histopathological appearance. The tumor cells are more elongated and less 166
densely cellular in Embryonal type. In alveolar type the tumor cells tend to be smaller 167
and rounder, often with a denser cellularity, and are so named because of their 168
resemblance to the appearance of the small air sacs in the lungs (the 169
"alveoli").Rhabdomyosarcomas have very poor prognosis with 5 year survival rate of 170
80% for localised disease and 5% for metastatic disease. 171
These cases have a great challenge in treatment because of the rarity of the disease 172
and absence of clinical trials and standard treatment guidelines. A lot of case reports 173
published showed that most patients expired within 5 years of the diagnosis. 174
Neoadjuvant chemotherapy was found to extend the survival of these patients [5]. 175
Postoperative chemotherapy is advised for all patients. Various chemotherapy 176
regimens are applied for these patients like Vinorelbine and Cyclophosphamide, 177
Gemcitabine and Docetaxel, Rapamycin, Topotecan and Vincristine [3]. Our patient 178
received Ifosfamide and carboplatin for 1 cycle, but unfortunately patient suddenly 179
died of acute respiratory distress and pleural effusion. 180
With this case report we would like to stress the aggressive nature of this disease 181
and the necessity for early detection of the disease. A multidisciplinary approach is 182
needed in managing these patients as there is no protocol available. An extensive 183
work-up is essential because these tumours have the potential to grow rapidly. PET 184
CT scan may be of beneficial in these patients before surgery [2] and may help in 185
diagnosing the metastasis before taking up for hepatectomy. 186
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CONCLUSION 191
The poor prognosis and early death of most cases reported so far implies the 192
necessity of early detection and evaluation of the disease. Surgical resection along 193
with chemotherapy provides improved survival only if given in the early stage of the 194
disease. 195
196
CONFLICT OF INTEREST 197
Authors declare no conflict of interest 198
199
REFERENCES 200
1. Hepatic rhabdomyosarcoma in an adult: A rare primary malignant liver tumor. 201
Case report and literature review. Schoofs G, Braeye L, Vanheste R, 202
Verswijvel G, Debiec-Rychter M, Sciot R. Acta Gastroenterol Belg 2011 203
Dec;74(4):576-81. 204
2. Primary embryonal rhabdomyosarcoma of the liver in a young male. Haider N, 205
Nadim MS, Piracha MN. J Coll Physicians Surg Pak 2013 Oct;23(10):750-1. 206
3. Management of undifferentiated embryonal sarcoma of the liver in children: A 207
case series and management review. Geel JA, Loveland JA, Pitcher GJ, 208
Beale P, Kotzen J, Poole JE. S Afr Med J 2013 Jun 27;103(10):728-31. 209
4. Undifferentiated Embryonal sarcoma of liver- Combination treatment by 210
surgery and chemotherapy. Dae-Yeon kim, Ki- Hong Kim, Seoul Korea, 211
presented in 34th annual meeting of pacific associations of paediatric 212
surgeons, Japan. 213
5. Almogy G, Lieberman S, Gips M, Pappo O, Edden Y, Jurim O, Simon Slasky 214
B, Uzieli B, Eid A: Surgical outcomes of surgical resections for primary liver 215
sarcomas in adults: Results from a single center. Eur J Surg Oncol 216
2004;30:421-427. 217
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TABLES 219
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FIGURE LEGENDS 223
Figure 1: CECT abdomen showing heterogenous mass lesion in right lobe of liver. 224
Figure 2: Coronal view of CECT abdomen showing the lesion occupying the entire 225
right lobe. 226
Figure 3: Intraop picture showing ruptured capsule of tumor. 227
Figure 4: Rhabdomyosarcoma – During surgery – Tying the Falciparum ligament. 228
Figure 5: Parenchymal resection using Harmonic Scalpel. 229
Figure 6: Post operative specimen of Rhabdomyosarcoma liver. 230
Figure 7: (a) HPE showing pleomorphic nuclei and spindle cells. (b) HPE showing 231
liver parenchyma with mitotic figures. 232
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FIGURES 234
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Figure 1: CECT abdomen showing heterogenous mass lesion in right lobe of liver. 236
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Figure 2: Coronal view of CECT abdomen showing the lesion occupying the entire 240
right lobe. 241
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Figure 3: Intraop picture showing ruptured capsule of tumor. 245
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Figure 4: Rhabdomyosarcoma – During surgery – Tying the Falciparum ligament. 248
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Figure 5: Parenchymal resection using Harmonic Scalpel. 252
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Figure 6: Post operative specimen of Rhabdomyosarcoma liver. 255
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a 257
b 258
Figure 7: (a) HPE showing pleomorphic nuclei and spindle cells. (b) HPE showing 259
liver parenchyma with mitotic figures. 260